The search for a physiologic marker of Machado-Joseph disease

Machado-Joseph disease is a dominantly inherited, multisystem, degenerative disorder that lacks a proven genetic marker. Peripheral nerve conduction-refractory period, sensory evoked potentials, and quantified oculomotor recordings were studied in nine patients affected with this disease to look for a potential physiologic marker. Only the oculomotor measurements of saccade and smooth pursuit gain were consistently abnormal in all patients. Identical eye movement recordings in 12 asymptomatic individuals at risk for Machado-Joseph disease revealed findings typical of affected patients in only 1 individual. Quantified oculomotor studies may contribute to the early confirmation of the disease, primarily in individuals at risk with minor or equivocal neurologic signs.     

Large volume injection and infiltration system

Summary A system which can be used for injection or infiltration of large volumes of fluid is described. This consists of a syringe which fills automatically from a reservoir through an inlet/outlet valve.     

Dermatologic changes associated with interleukin 2 administration

We have prospectively evaluated the skin changes that occurred in ten patients who were undergoing immunotherapy with interleukin 2 (IL-2) and autologous lymphokine-activated killer cells to treat cancer. Serial skin biopsy specimens were obtained before therapy (baseline), during IL-2 administration, and during IL-2/lymphokine-activated killer cell infusion. All patients developed an eruption that was characterized by macular erythema, with burning and pruritus of the skin. It began after two or three days of IL-2 infusion and was usually localized to the head and neck; it occasionally became generalized (ie, erythroderma). The eruption resolved with desquamation within 48 to 72 hours after cessation of infusion of IL-2. Histologically, the changes were not specific. The only consistent immunohistological finding noted was the presence of DR+/Leu-4+ lymphoid cells surrounding blood vessels in the papillary dermis, with fewer of these cells in the epidermis. There was no difference between the clinical or histological features of the eruption that occurred with IL-2 alone and that which occurred with IL-2 and lymphokine-activated killer cell infusion, suggesting that the cutaneous effects were mediated by IL-2 alone.     

Family Stress and Functioning in Children: the Moderating Effects of Children''s Beliefs About Their Control Over Parental Conflict

While factors such as gender and SES have been studied as moderators of stress for children, their perceptions of control have received little attention. In the current study, children's domain-specific perceptions of their control during marital conflict were investigated as potential moderators of the impact of family stress on children's behavior problems, and perceptions of competence. Ninety-four children aged 6-12 years and mothers from families ranging in level of parental conflict from nondiscordant to discordant to physically violent were interviewed. Multiple regression analyses revealed that higher levels of conflict control beliefs acted as compensatory moderators of stress, being associated with lower levels of problem behaviors across stress levels. However, higher conflict control beliefs acted as vulnerability moderators with regard to children's perceptions of competence.     

Effect of link protein concentration on articular cartilage proteoglycan aggregation

Previous work has shown that alterations in proteoglycan aggregates are among the first changes detected with aging, disuse, and degeneration of articular cartilage, yet the cause or causes of these alterations remain unknown. To determine if differences in link protein concentration can explain alterations in the assembly, size, and stability of articular cartilage proteoglycan aggregates, we isolated proteoglycan monomer (aggrecan) and link protein from adult bovine articular cartilage and then assembled proteoglycan aggregates from aggrecan and 0.8% hyaluronan relative to aggrecan weight, in the presence of 0, 2, 4, 6, 8, 10, 15, and 20% concentrations of link protein relative to aggrecan weight. We determined the amount, sedimentation coefficient, and stability of the aggregates by analytical ultracentrifugation and measured their dimensions by electron microscopy with use of the monolayer technique. Increased aggregate size, as determined by ultracentrifugation, was directly correlated with an increased number of aggrecans per aggregate and with increased hyaluronan length, as determined by electron microscopy. The concentration of link protein significantly influenced aggregation: concentrations of 6–8% produced maximum aggregation, aggregate stability, and uniformity of aggrecan spacing; concentrations greater than 10% led to the formation of superaggregates (aggregates with sedimentation velocities greater than 100 S that may result from linking two or more hyaluronan filaments) but decreased aggregate stability; and concentrations of less than 4% link protein significantly decreased aggregation, the size and stability of aggregates, and the regularity of aggrecan spacing. The latter observations suggest that a decline in the concentration of link protein could decrease the organization and stability of the articular cartilage matrix.     

Chondroblastoma of the rib. A case report and review of the literature

Chondroblastoma, a rare bone tumor usually found in the epiphyseal region of long bones, appeared in the rib of a 49-year-old man. Review of the literature reveals that the most common locations for this tumor are the knee and proximal humerus. The most common age group is the second decade. Eighteen cases of chondroblastoma of the rib have been reported in the literature; the patients were older than typical patients with epiphyseal chondroblastoma and had an excellent prognosis after resection of the tumor.     

T cell subsets mediating rejection of established fetal pancreas grafts and failure to observe graft adaptation

The present study has attempted to elucidate the cellular mechanisms by which long-term established fetal pancreas allografts are rejected. We used an experimental model in which H-2b nude mice were made hyperglycemic by streptozotocin treatment and then engrafted with allogeneic fetal pancreas grafts. These grafts were functional in that engrafted animals returned to near normoglycemia while all animals left unengrafted subsequently died. The fetal pancreas grafts were allowed to reside in the immunoincompetent nude host for 6-9 months prior to T cell reconstitution, at which time animals were reconstituted with either negatively selected CD4+ or CD8+ H-2b T cell subpopulations. We found that a 6-9 month residence in an immunoincompetent host did not lead to a change in the immunogenicity of fetal pancreatic grafts in that both CD4+ and CD8+ T cell subsets were capable of rejecting these long-term established fetal pancreas grafts. The finding that isolated CD8+ spleen T cell subpopulations, which are only activated by antigen-presenting cells of donor origin bearing MHC class I alloantigen, were capable of effecting graft rejection suggested that APC of donor origin persisted in these long-term fetal pancreas allografts.     

Cemented and ingrowth fixation of the Miller-Galante prosthesis. Clinical and roentgenographic comparison after three- to six-year follow-up studies

One hundred thirty-nine cemented and 132 cementless Miller-Galante total knee prostheses were followed between three and six years (average, 43-44 months). The fixation technique was based on patient age, bone quality, and ability to delay full-weight bearing. Clinical follow-up studies were possible on 116 cemented knees. Fifteen knees were lost because of death before the three-year follow-up study, and eight knees required component removal. One hundred twenty-three cementless knees were available for clinical follow-up studies; there were three deaths, and six failures required component removal. No cemented failure was due to fixation, and three cementless failures were due to lack of tibial ingrowth in two and pain of undetermined etiology in one. Preoperative knee scores were slightly significant with cemented knees averaging 48 points and cementless knees averaging 52 points. A similar significant difference was maintained at the final follow-up study. No significant differences were noted for pain, limp, or support scores. Average range of motion was similar in the two groups. Radiolucent lines about the femoral component were rare. Cementless tibial radiolucencies were partial in up to 20% of examined zones, and complete tibial tray radiolucency was seen in only three patients. No correlation between radiolucency and knee scores was seen.     

Botrocetin agglutination of rat megakaryocytes: a rapid method for megakaryocyte isolation.

Botrocetin isolated from the venom of Bothrops jaracara has been shown by others to induce binding of von Willebrand Factor to glycoprotein Ib and thereby produce platelet agglutination in a wide range of animal species. We have found that botrocetin also facilitates the agglutination of megakaryocytes and have used this property to develop a method to isolate megakaryocytes from rat bone marrow. When botrocetin is added to a mixture of rat bone marrow, rat platelets, and rat plasma, coagglutination of megakaryocytes and platelets occurs. The agglutinated complexes, containing > 95% of the megakaryocytes, may then be separated from the remaining marrow cells by filtration. Megakaryocytes account for 39% (range 30%-48%) of the isolated cells and 83% (range 77%-88%) of the isolated cell mass. This method allows the virtually complete removal of megakaryocytes from bone marrow as well as their isolation to a high degree of purity. It should provide a useful, inexpensive, general method for the rapid isolation of megakaryocytes from multiple, small marrow samples from a wide range of species.